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Although the association of Castleman’s disease with autoimmune connective tissue disease is rare, few patients have been reported with systemic lupus erythematosus, rheumatoid arthritis, myasthenia gravis, and Sjögren’s syndrome. We present a patient fulfilling the American College of Rheumatology criteria for systemic lupus erythematosus, who responded to medical therapy but flared with generalized lymphadenopathy proved to be Castleman’s disease.